Case Of Desmoplastic Fibroma Research Articles

DESMOPLASTIC FIBROMA OF GNATHIC BONES: A SYSTEMATIC REVIEW

Objective To analyze the available data regarding desmoplastic fibroma (DF) into a systematic review to integrate the demographic, clinical and histopathological features, treatment, and recurrence of this condition. Study Design The present study was conducted based on PRISMA statement. An electronic search was undertaken in August 2021 in four databases. The eligibility criteria were restricted to case reports, case series. and cross-sectional studies. Results Eighty-one articles met the eligibility criteria, yielding 125 cases of DF. Women (60.8%) were more affected, with a mean age of 19.1 years (SD±16.7). In relation to the anatomical site, 69.4% lesions were found in posterior mandible, with a bulging tumor clinical appearance. Most lesions were asymptomatic (85.9%) with a mean evolution time of 11 months. Radiologically, the tumor is described as a radiolucent (92,4%) multilocular (70.5%) lesion, presenting defined borders (35.5%) as well as causing bone expansion (28.2%). Resection (53.1%) and enucleation (14.3%) were the treatment choice in most cases. Recurrence was observed in 11.6% of the cases, with resection being the treatment of choice (100%). Conclusion DF is a rare bone tumor with a challenging diagnosis and treatment. This study provides information that could be helpful for the diagnosis and management of this condition. To analyze the available data regarding desmoplastic fibroma (DF) into a systematic review to integrate the demographic, clinical and histopathological features, treatment, and recurrence of this condition. The present study was conducted based on PRISMA statement. An electronic search was undertaken in August 2021 in four databases. The eligibility criteria were restricted to case reports, case series. and cross-sectional studies. Eighty-one articles met the eligibility criteria, yielding 125 cases of DF. Women (60.8%) were more affected, with a mean age of 19.1 years (SD±16.7). In relation to the anatomical site, 69.4% lesions were found in posterior mandible, with a bulging tumor clinical appearance. Most lesions were asymptomatic (85.9%) with a mean evolution time of 11 months. Radiologically, the tumor is described as a radiolucent (92,4%) multilocular (70.5%) lesion, presenting defined borders (35.5%) as well as causing bone expansion (28.2%). Resection (53.1%) and enucleation (14.3%) were the treatment choice in most cases. Recurrence was observed in 11.6% of the cases, with resection being the treatment of choice (100%). DF is a rare bone tumor with a challenging diagnosis and treatment. This study provides information that could be helpful for the diagnosis and management of this condition.

MYOFIBROBLASTIC LESIONS IN THE ORAL CAVITY: IMMUNOHISTOCHEMICAL ANALYSIS

Objective To characterize immunohistochemically a group of oral myofibroblastic lesions (MLs). Study Design MLs incorporated during the period of 1997-2014 were retrieved from the files and records of the Department of Oral Pathology. Cases of myofibroma (MF), nodular fasciitis (NF), desmoplastic fibroma (DF), and 2 myofibroblastic sarcoma (MS) were stained with antibodies against alpha-smooth muscle actin (α-SMA), H-caldesmon, vimentin, desmin, β-catenin, anaplastic lymphoma protein kinase (ALK-1), and Ki-67. Results Nineteen of 22 cases of MF, 2/5 NF, 1/10 DF, and 1/2 MS were positive for α-SMA. All cases of MF, NF, DF, and MS were positive for vimentin. One-half of MS cases were positive for desmin. Six out of 10 cases of DF were positives for β-catenin. Two cases of MF were positive for ALK-1. All cases of MF, NF, DF, and MS were negative for H-caldesmon. The Ki-67 labeling index was 10% and 8/22 cases of MF, 3/5 cases of NF, and 2/2 cases of MS exhibited a rate of ≥ 10% for this marker. Conclusions The application of this panel of markers can help the diagnosis of oral MLs, in particular the distinction between fibroblastic, myofibroblastic, and muscle cells proliferation.

DESMOPLASTIC FIBROMA OF THE MANDIBLE: A DIAGNOSTIC CHALLENGE

Desmoplastic fibroma (DF) is a rare benign myofibroblastic tumor with a locally aggressive behavior and high recurrence rate. We describe a case of DF in a 27-year-old woman presenting a painful, soft exophytic lesion on the mandible with approximately 7 months of evolution. Panoramic radiograph showed a radiolucent unilocular lesion with sclerotic borders involving teeth 44, 45, and 47. Clinical diagnosis was ossifying fibroma, and incisional biopsy was performed. Histopathologic analysis revealed a neoplasm characterized by proliferation of round, ovoid, and spindle-shaped mesenchymal cells with indistinct borders and no atypia. Intense deposition of collagen fibers, interspersed with areas of low-to-moderate cellularity, were also found. Neoplastic cells showed immunopositivity for α–smooth muscle actin, S-100, synaptophysin, neuron-specific enolase, and Ki-67 but no immunoexpression of desmin. The patient was referred for tumor excision. This case reinforces the importance of associating clinical, radiographic, histopathologic, and immunohistochemical findings for the correct diagnosis and management of DF.

下顎前歯部に生じた類腱線維腫の1例

下顎前歯部に生じた類腱線維腫の1例

Desmoplastic fibroma of the mandible: report of three cases with a review of literature

The desmoplastic fibroma (DF) is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. We present three cases of DF occurring in the mandible: the first in a 13 year old female involving the body of the mandible in the region of #27-#28, the second in a 57 year old female with a lesion apical to tooth #30, and the third in a 20-year-old female involving the left posterior mandible. The clinical, histologic, immunohistochemical and radiographic features of this rare neoplasm are discussed. The challenges encountered in establishing an accurate diagnosis due to significant microscopic overlap with other spindle cell lesions are also detailed. We performed immunohistochemical stains including vimentin, smooth muscle actin, S-100 protein, 2-catenin, HHF-35 and proliferation marker, Ki-67 for all 3 cases. The potential for misdiagnosis is high, especially in the early lesions, since immunohistochemistry has been reported in literature to be inconsistent when differentiating DFs from other spindle cell lesions. A comparative review of DF and similar lesions with current considerations in treatment and prognosis is presented.

下顎枝に生じた類腱線維腫の1例

下顎枝に生じた類腱線維腫の1例

Desmoplastic fibroma of the rib with cystic change: a case report and literature review

Desmoplastic fibroma (DF) is a rare, locally aggressive, solitary tumor microscopically composed of well-differentiated myofibroblasts with abundant dense collagen deposition. The most common sites are the long tubular bones and mandible. To our knowledge, only five cases of DF in the ribs have been reported. Here, we report a case of DF in this rare location with unusual radiological findings. A 40-year-old man presented with a 4-year history of swelling of the right chest wall. Radiographs revealed a mass at the right 9th rib, and computed tomography demonstrated a mass of 14 × 12 × 8cm at the right 9th rib with expanded cystic change and marked calcification that appeared to have arisen from the bone. Open biopsy suggested DF. Total excision was performed, and the chest wall was reconstructed. The surgical specimen was a yellowish tumor with multilocular cystic change containing a viscous liquid. The tumor was composed of a proliferation of less-atypical spindle-shaped cells in a collagenous background. The cystic change was observed in the extra-osseous lesion. No β-catenin cytoplasmic/nuclear accumulation was detected, and no β-catenin or GNAS genetic mutations were detected. A final diagnosis of DF was made on the basis of the pathological and radiological findings. The patient was successfully treated with total excision of the tumor with no evidence of recurrence 6months after surgery.

A case of desmoplastic fibroma in the mandible

A case of desmoplastic fibroma in the mandible

Desmoplastic fibroma of the pediatric cranium: case report and review of the literature

Desmoplastic fibromas are primary bone tumors that seldom occur in the cranial bones. Furthermore, reports of desmoplastic fibromas of the skull in children are exceedingly rare. Although desmoplastic fibromas are histologically benign, they are locally aggressive and have a propensity to reoccur. Their radiographic appearance may mimic other more common central nervous system and bone neoplasms. There are only 19 reported cases of desmoplastic fibroma of the cranium in the literature, and only seven occurred in the pediatric age group. We present a case report of an 11-year-old female patient with a desmoplastic fibroma of the parieto-occipital region and review the literature. An 11-year-old female presented to the craniofacial clinic complaining of intermittent pain and a soft mass in the occipital region. There was a distant history of trauma to the region that did not require medical intervention. Computed tomography imaging revealed a lytic bone lesion overlying the sagittal sinus in the parieto-occipital region. Surgical resection with wide margins and immediate autologous reconstruction was performed. Pathological analysis revealed a desmoplastic fibroma. At 4 months of follow-up, no recurrence has been noted. Desmoplastic fibroma of the cranium is rare. Complete surgical resection with careful follow-up is the treatment of choice.

Maxillary desmoplastic fibroma with initial symptoms suggestive of sinusitis

Desmoplastic fibroma (DF) is a benign intra-osseous neoplasm characterized by the formation of abundant collagen fibers. It arises most commonly in the jaws and exhibits local aggressiveness and high recurrence rates after local resection. An uncommon case of expansive DF involving the right maxilla, maxillary sinus, and inferior orbital wall of a 49-year-old man whose initial symptoms were similar to acute sinusitis is presented, and the criteria for diagnosis and clinical management are discussed.

Desmoplastic fibroma of mandible

Desmoplastic fibroma is a rare benign intraosseous tumor with locally aggressive behavior. The mandible is the most frequent site of involvement. It causes bone destruction with a tendency to invade the soft-tissues if untreated. It has a high propensity for local recurrence if treated inadequately. We report a case of desmoplastic fibroma of the mandible treated with a wide margin of excision.

Desmoplastic fibroma with malignant transformation

Desmoplastic fibroma (DF) is a rare neoplasm of bone, showing infiltrative and locally aggressive nature. Here, we report a case of DF with an unusual histology arising in a 41-year-old female in the left distal femur, which was detected by plain x-ray as an osteolytic lesion and by magnetic resonance imaging as a well-demarcated mass with endosteal scalloping. Pathologically, the tumor was composed mainly of bland-looking spindle cells in abundant collagenous stroma, accompanied with areas of myxofibrosarcomatous and malignant fibrous histiocytomatous components. These histologically different areas were admixed with each other. The array-based comparative genomic hybridization study on the histologically different areas showed some specific gained or lost loci according to their histologic features. The specific genetic events and the histologic features of this case might represent the malignant transformation of DF.

Desmoplastic fibroma of the skull in an infant

A 1-year-old girl presented with a 10-month history of progressive protuberance of the left frontal skull. Magnetic resonance imaging and computed tomography demonstrated a large osteolytic interosseous mass extending to the frontal sinus and temporal base without any intracranial invasion. A fronto-temporo-parietal craniectomy of the outer skull table and excision of an interosseous tumor resulted in local dural exposure in the temporal area that was covered by cranioplasty. Pathological examination identified desmoplastic fibroma (DF) of the skull. The patient's cranial asymmetry was improved without recurrence of the tumor up to the 12th month after excision. In the literature, 11 cases of DF of the skull have been reported, two of which have involved children (one an infant). We report the second known infantile case of DF of the skull.

MR FINDINGS OF DESMOPLASTIC FIBROMA OF THE SPINE. A case report

We report on the MR imaging findings in a case of spinal desmoplastic fibroma (DF). DF of the bone is a rare, locally aggressive tumor. It is commonly located in long bones, pelvis or mandible. DF involving the spine is extremely rare and difficult to distinguish from other bony lesions such as giant cell tumor, chordoma and fibrous dysplasia of the spine. This case of DF of the spine showed MR findings similar to those of DF arising in the metaphysis of a long bone.

MR findings of desmoplastic fibroma of the spine: A case report

We report on the MR imaging findings in a case of spinal desmoplastic fibroma (DF). DF of the bone is a rare, locally aggressive tumor. It is commonly located in long bones, pelvis or mandible. DF involving the spine is extremely rare and difficult to distinguish from other bony lesions such as giant cell tumor, chordoma and fibrous dysplasia of the spine. This case of DF of the spine showed MR findings similar to those of DF arising in the metaphysis of a long bone.

Desmoplastic Fibroma in the Thoracic Spine

Desmoplastic fibroma of the spine is an extremely rare, benign tumor. So far, only 7 cases have been reported in the world. We have treated 1 case of desmoplastic fibroma in the thoracic spine. Due to insufficient removal of the tumor, local recurrence was observed, and a second surgery posteriorly and anteriorly was performed for complete removal 8 years following the first operation. After resection of the tumor, Luque instrumentation was used to reconstruct the destroyed spine. At present, 7 years after the second operation, there are no signs or symptoms. Therefore, complete resection of the tumor is most important in the treatment, and rigid spinal instrumentation and bone grafts must be used after removing the tumor in spine cases.

CT-GUIDED BIOPSY OF METASTATIC SACRAL TUMORS

Between June 8, 1985 and January 29, 1986, the authors performed five CT-guided needle biopsies of suspicious sacral lesions. In each of the five cases, these biopsies allowed the acquisition of specimens suitable for definitive pathologic diagnosis. Three cases of metastatic adenocarcinoma, one case of desmoplastic fibroma, and one case of chronic inflammatory changes were noted. In the case involving chronic inflammatory changes, subsequent open biopsy was performed, confirming the inflammatory changes and absence of neoplasm. The authors conclude that percutaneous CT-guided biopsy of sacral lesion can be performed safely and rapidly, and allows accurate diagnosis and treatment of sacral lesions.

Desmoplastic fibroma of bone: radiographic analysis.

Desmoplastic fibroma (DF) of bone is a rare, nonmetastasizing but locally aggressive tumor that has been discussed infrequently in the radiology literature. The radiographs from 107 previously published cases of DF and seven cases from the authors' institution were analyzed to better understand and define its radiographic characteristics. DF was most common in the mandible, pelvis, and femur. A geographic pattern of bone destruction, with a narrow zone of transition and nonsclerotic margins, was seen in 80 (96%) patients with intraosseous DF for whom radiographs were available (83 patients). Internal pseudotrabeculation was seen in 76 (91%). Although widening of the host bone due to gradual apposition of periosteal new bone was common, occurring in 74 (89%) patients, distinct periosteal new bone occurred in only two (2%) patients with DF of intraosseous origin. The cortex was breached in 23 (28%) patients. Three cases of DF arising in the periosteum were identified and were differentiated radiographically from desmoid tumors of intraosseous or soft-tissue origin.

Desmoplastic fibroma of the mandible case report

We present a case of desmoplastic fibroma in a child. The microscopic findings of desmoplastic fibroma of bone closely resemble that of the soft tissue desmoid tumor. Particularly, the agressive fibromatosis which occures peripherally and that proliferates to cortical bone is very similar to this tumor.